Ventricular septal defect: an unusual cause of paradoxical low-gradient aortic stenosis

<span class="paragraphSection">A 57-year-old patient was referred for cardiac evaluation before non-cardiac surgery. Doppler echocardiography showed a restrictive perimembranous ventricular septal defect (VSD) (arrowheads, see Supplemenatry data onlineSupplemenatry data online, <span style="font-style:italic;">Video S1, Panels A </span>and <span style="font-style:italic;">B</span>). Left ventricular (LV) ejection fraction was 75%. Aortic valve was severely calcified (<span style="font-style:italic;">Panel C</span>), with a 24 mmHg mean pressure gradient and a 3.2 m/s maximal velocity. Aortic time velocity integral (TVI) was 84 cm (<span style="font-style:italic;">Panel D</span>). By continuity equation, aortic valve area (AVA) was 1.13 cm² using Doppler LV outflow tract (LVOT, stroke volume 95 mL) and 1.06 cm² using biplane Simpson method (stroke volume 89 mL), respectively. However, in the setting of a perimembranous VSD, the assumption of both the continuity equation (similar flow in the LVOT and across the aortic valve) and the Gorlin formula (similar flow in the right heart and across the aortic valve) are inherently unmet. Therefore, cardiac catheterization was performed to determine the systemic flow and to quantify the shunt. Pulmonary pressures were normal. Qp/Qs was 1.3 and AVA was 0.80 cm² using the combination of oximetry-derived systemic stroke volume value (72 mL) and simultaneous trans-aortic continuous wave Doppler for aortic TVI assessment. Transoesophageal echocardiography revealed a bicuspid aortic valve, with a 0.82 cm² AVA by planimetry obtained using three-dimensional reconstruction (Supplementary dataSupplementary data online, <span style="font-style:italic;">Video 2, Panels E</span> and <span style="font-style:italic;">F</span>). Aortic valve calcium score was 3900 AU by multidetector computed tomography (MDCT).</span>

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